Plummer-Vinson syndrome overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
The Plummer-Vinson syndrome, also called Paterson-Brown-Kelly syndrome or sideropenic dysphagia is a disorder linked to severe, long-term iron deficiency anemia, which causes swallowing difficulty (dysphagia) due to web-like membranes of tissue growing in the throat (esophageal webs). [1] The disease is named after two Americans, the physician Henry Stanley Plummer, and the surgeon Porter Paisley Vinson. [2][3][4]
It is also sometimes called "Kelly-Paterson syndrome", after Adam Brown-Kelly and Donald Ross Paterson.[2][5][6]
Historical Perspective
Plummer-Vinson syndrome was first discovered by Henry Plummer an American internist, in a case series of patients with long-standing iron deficiency anemia, dysphagia and spasm of the upper esophagus without anatomic stenosis in his article "Diffuse dilatation of the esophagus without anatomic stenosis." In the year 1919, Porter Paisley Vinson an American surgeon at the Mayo Clinic further described Plummer-Vinson syndrome in his article "A case of cardiospasm with dilatation and angulation of the esophagus." He reported a case of angulation of esophagus and attributed his findings to be consistent as described by Henry Plummer. In the year 1919, Donald Ross Patterson and Adam Brown Kelly, both British otolaryngologist described the characteristic clinical features of Plummer-Vinson syndrome in their article "A clinical type of dysphagia" and "Spasm at the entrance of the esophagus" respectively.
Classification
There is no established system for the classification of Plummer-Vinson syndrome.
Pathophysiology
Causes
Differentiating Plummer-Vinson syndrome overview from Other Diseases
Epidemiology and Demographics
Plummer-Vinson syndrome is a rare disease and the data pertaining to incidence and prevalence is not evidently available. Overall improvement in nutritional status with better medical care has markedly reduced the number of cases of Plummer-Vinson syndrome. However, individuals of any age groups may develop Plummer-Vinson syndrome and it is most commonly seen in the age group of 40-70 years. Plummer-Vinson syndrome usually affects individuals of the caucasian race. Females are commonly affected than males with female to male ratio of 4:1. The majority of Plummer-Vinson syndrome cases are reported in Scandinavian countries or north European countries.
Risk Factors
Screening
Natural History, Complications, and Prognosis
If left untreated, patients of Plummer-Vinson syndrome may progress to develop fatigue, dyspnea on exertion, esophageal strictures, and malignant lesions of the mouth and oral cavity. Common complications of Plummer-Vinson syndrome include hypopharyngeal cancer, esophageal cancer and malignant lesions of oral mucosa. Depending on the extent of Plummer-Vinson syndrome at the time of diagnosis, the prognosis may vary. Prognosis is generally good for patients who receive treatment. Iron replacement therapy and dilatation of esophageal web leads to rapid reversal of symptoms.
Diagnosis
Diagnostic Criteria
History and Symptoms
Physical Examination
Laboratory Findings
Laboratory findings consistent with the diagnosis of Plummer-Vinson syndrome include presence of iron deficiency anemia. Patients suspected of Plummer-Vinson syndrome should be tested with complete blood count (CBC), iron studies, peripheral smear, stool test for occult blood, blood lead levels and bone marrow biopsy for stainable iron.
Imaging Findings
Videofluoroscopy may be helpful in the diagnosis of Plummer-Vinson syndrome. Videofluoroscopy is done in patients with normal barium esophagogram who have a high probability of Plummer-Vinson syndrome. Videofluoroscopy is superior to barium esophagogram and has the ability to detect small esophageal webs resulting from insignificant mucosal and submucosal foldings which may otherwise go undiagnosed.
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Prevention
References
- ↑ Novacek G (2006). "Plummer-Vinson syndrome". Orphanet J Rare Dis. 1: 36. doi:10.1186/1750-1172-1-36. PMID 16978405.
- ↑ 2.0 2.1 Template:WhoNamedIt
- ↑ H. S. Plummer. Diffuse dilatation of the esophagus without anatomic stenosis (cardiospasm). A report of ninety-one cases. Journal of the American Medical Association, Chicago, 1912, 58: 2013-2015.
- ↑ P. P. Vinson. A case of cardiospasm with dilatation and angulation of the esophagus. Medical Clinics of North America, Philadelphia, PA., 1919, 3: 623-627.
- ↑ A. B. Kelly. Spasm at the entrance of the esophagus. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 34: 285-289.
- ↑ D. R. Paterson. A clinical type of dysphagia. The Journal of Laryngology, Rhinology, and Otology, London, 1919, 24: 289-291.